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  • 产品名称:MYOT, Blocking Peptide

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简单介绍:
MYOT, Blocking Peptide
详情介绍:
Product Name

MYOT, Blocking Peptide

Full Product Name

MYOT Antibody (Center) Blocking Peptide

Product Synonym Names
Myotilin; 57 kDa cytoskeletal protein; Myofibrillar titin-like Ig domains protein; Titin immunoglobulin domain protein; MYOT; TTID
Product Gene Name

MYOT blocking peptide

[Similar Products]
Product Synonym Gene Name
TTID[Similar Products]
Antibody/Peptide Pairs
MYOT peptide (MBS9222394) is used for blocking the activity of MYOT antibody (MBS9207572)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
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OMIM
159000
3D Structure
ModBase 3D Structure for Q9UBF9
Form/Format
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
Cellular Location
Cell membrane, sarcolemma. Cytoplasm, cytoskeleton. Cytoplasm, myofibril, sarcomere, Z line Note: Sarcomeric, also localized to the sarcolemma. Colocalizes with MYOZ1 at the Z-lines in skeletal muscle
Tissue Location
Expressed in skeletal muscle (at protein level). Expressed in skeletal muscle, heart, bone marrow and thyroid gland.
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of MYOT blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for
MYOT blocking peptide
Component of a complex of multiple actin cross-linking proteins. Involved in the control of myofibril assembly and stability at the Z lines in muscle cells.
NCBI/Uniprot data below describe general gene information for MYOT. It may not necessarily be applicable to this product.
NCBI GI #
311033402
NCBI GeneID
9499
NCBI Accession #
Q9UBF9.2 [Other Products]
UniProt Primary Accession #
Q9UBF9 [Other Products]
UniProt Secondary Accession #
A0A4R6; B4DT79[Other Products]
UniProt Related Accession #
Q9UBF9[Other Products]
Molecular Weight
35,147 Da
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NCBI Official Full Name
Myotilin
NCBI Official Synonym Full Names
myotilin
NCBI Official Symbol
MYOT  [Similar Products]
NCBI Official Synonym Symbols
MFM3; TTID; TTOD; LGMD1; LGMD1A
  [Similar Products]
NCBI Protein Information
myotilin
UniProt Protein Name
Myotilin
UniProt Synonym Protein Names
57 kDa cytoskeletal protein; Myofibrillar titin-like Ig domains protein; Titin immunoglobulin domain protein
Protein Family
Myotilin
UniProt Gene Name
MYOT  [Similar Products]
UniProt Synonym Gene Names
TTID  [Similar Products]
UniProt Entry Name
MYOTI_HUMAN
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NCBI Summary for MYOT
This gene encodes a cystoskeletal protein which plays a significant role in the stability of thin filaments during muscle contraction. This protein binds F-actin, crosslinks actin filaments, and prevents latrunculin A-induced filament disassembly. Mutations in this gene have been associated with limb-girdle muscular dystrophy and myofibrillar myopathies. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined.[provided by RefSeq, Oct 2008]
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UniProt Comments for MYOT
MYOT: Component of a complex of multiple actin cross-linking proteins. Involved in the control of myofibril assembly and stability at the Z lines in muscle cells. Defects in MYOT are the cause of limb-girdle muscular dystrophy type 1A (LGMD1A). LGMD1A is an autosomal dominant degenerative myopathy with onset within a mean age of 28 years. LGMD1A is characterized by progressive skeletal muscle weakness of the hip and shoulder girdles, later progressing to include distal weakness, as well as a distinctive dysarthric pattern of speech. Affected muscle exhibits disorganization and streaming of the Z-line. Defects in MYOT are the cause of myopathy myofibrillar type 3 (MFM3). A neuromuscular disorder characterized by progressive skeletal muscle weakness greater distally than proximally, tight heel cords, hyporeflexia, cardiomyopathy and peripheral neuropathy in some patients. Affected muscle exhibits disorganization and streaming of the Z-line, presence of large hyaline structures, excessive accumulation of myotilin and other ectopically expressed proteins and prominent congophilic deposits. Defects in MYOT are the cause of spheroid body myopathy (SBM). SBM is an autosomal dominant form of myofibrillar myopathy (MFM), characterized by slowly progressing proximal muscle weakness and dysarthric nasal speech. There is no evidence of cardiomyopathy. Muscle biopsy shows spheroid bodies within the type I muscle fibers. Belongs to the myotilin/palladin family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Cytoskeletal

Chromosomal Location of Human Ortholog: 5q31

Cellular Component: actin cytoskeleton; Z disc

Molecular Function: alpha-actinin binding; protein binding; structural constituent of muscle

Biological Process: muscle contraction

Disease: Muscular Dystrophy, Limb-girdle, Type 1a; Myopathy, Myofibrillar, 3; Myopathy, Spheroid Body
Research Articles on MYOT
1. Describe the first homozygous mutation in the myotilin gene leading to a novel, autosomal recessive subtype of myofibrillar myopathy (MFM).
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Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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